Immunglobulin-A Mediated Vasculitis in |A 9 Years-Old Boy |Biomedgrid

IgA Vasculitis (IgAV), formerly known as Schönlein-Henoch purpura, is a leukocytoclastic vasculitis affecting small vessels, especially the skin, gastrointestinal tract, and glomeruli, with predominantly IgA-containing immune complex deposits, often accompanied by arthralgias and/or arthritis. Clinically, the disease manifests on the skin with palpable purpura, abdominal pain, intussusception, nephritis, joint swelling or pain. Schönlein first described purpura-associated arthritis in 1837, while Enoch reported the combination of nephritis with abdominal pain in 1874. The disease begins with a sudden onset of palpable purpura, typically on the feet, legs, and, occasionally, trunk and arms. The purpura begins in small urticarial districts that are palpable and sometimes hemorrhagic and confluent. Smaller groups of lesions may be added over days or weeks. Many patients also have fever and polyarthralgias with periarticular pain and swelling of the ankles, knees, hips, wrists, and elbows. Gastrointestinal symptoms are common and manifest as colicky abdominal pain, abdominal tenderness, and melena. Occasionally, intussusception occurs in children. Stool specimens may test positive for occult blood. Symptoms of immunoglobulin A-associated vasculitis usually resolve after about 4 weeks, and there is often at least one relapse after a disease-free interval of several weeks. In most patients, the disease heals without leaving serious damage. While rare, some patients may still develop chronic kidney disease. In adults, intussusception is rare and chronic kidney disease is more common than in children.